Mutant Huntingtin Gene-Dose Impacts on Aggregate Deposition, DARPP32 Expression and Neuroinflammation in HdhQ150 Mice

Huntington's disease (HD) is an autosomal dominant, progressive and fatal neurological disorder caused by an expansion of CAG repeats in exon-1 of the huntingtin gene. The encoded poly-glutamine stretch renders mutant huntingtin prone to aggregation. HdhQ150 mice genocopy a pathogenic repeat (∼...

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Autors principals: Young, Douglas, Mayer, Franziska, Vidotto, Nella, Schweizer, Tatjana, Berth, Ramon, Abramowski, Dorothee, Shimshek, Derya R., van der Putten, P. Herman, Schmid, Peter
Format: Artigo
Idioma:Inglês
Publicat: Public Library of Science 2013
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Research Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC3781050/
https://ncbi.nlm.nih.gov/pubmed/24086450
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0075108
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